Pendular Nystagmus in Patients With Peroxisomal Assembly Disorder

Abstract

The pendular nystagmus in these patients was due to their leukodystrophy and may have a similar pathogenesis to the oscillations seen in other disorders affecting central myelin.

Familial infantile bilateral striatal necrosis

“…Acquired horizontal pendular nystagmus, commonly occurring in congenital and acquired disorders of myelin, 16 appeared in Patient VI-14 at age 3.5 years and Patient VI-9 at age 5 years; optic atrophy later appeared in these two patients (age 10 and 9). The choreoathetosis progressed to hemiballismus combined with dystonic postures.…”

Familial infantile bilateral striatal necrosis

“…Acquired horizontal pendular nystagmus, commonly occurring in congenital and acquired disorders of myelin, 16 appeared in Patient VI-14 at age 3.5 years and Patient VI-9 at age 5 years; optic atrophy later appeared in these two patients (age 10 and 9). The choreoathetosis progressed to hemiballismus combined with dystonic postures.…”

A 6-month trial of memantine for nystagmus and associated phenomena in oculopalatal tremor

“…VOG horizontal and vertical eye tracings from each eye were analyzed offline with custom software ( http://faculty.washington.edu/jokelly/voganalysis ) . After manual artifact rejection (i.e., blinks, drop-outs, saccades, and erroneous gaze shifts), mean amplitude, velocity, and frequency of nystagmus were computed in a stable baseline tracing ( 11 , 12 ). The mean velocity was calculated using desaccaded tracings with an array size >2,000 points.…”

Eye movement disorders and neurological symptoms in late‐onset inborn errors of metabolism

“…Ocular motor abnormalities include hypometric saccades (particularly in the horizontal plane), saccadic intrusions (square‐wave jerks), and gaze‐evoked nystagmus . Pendular nystagmus can be observed . No curative treatment is available.…”

Familial infantile bilateral striatal necrosis

“…Acquired horizontal pendular nystagmus, commonly occurring in congenital and acquired disorders of myelin, 16 appeared in Patient VI-14 at age 3.5 years and Patient VI-9 at age 5 years; optic atrophy later appeared in these two patients (age 10 and 9). The choreoathetosis progressed to hemiballismus combined with dystonic postures.…”

A 6-month trial of memantine for nystagmus and associated phenomena in oculopalatal tremor

“…VOG horizontal and vertical eye tracings from each eye were analyzed offline with custom software ( http://faculty.washington.edu/jokelly/voganalysis ) . After manual artifact rejection (i.e., blinks, drop-outs, saccades, and erroneous gaze shifts), mean amplitude, velocity, and frequency of nystagmus were computed in a stable baseline tracing ( 11 , 12 ). The mean velocity was calculated using desaccaded tracings with an array size >2,000 points.…”

Eye movement disorders and neurological symptoms in late‐onset inborn errors of metabolism

“…Ocular motor abnormalities include hypometric saccades (particularly in the horizontal plane), saccadic intrusions (square‐wave jerks), and gaze‐evoked nystagmus . Pendular nystagmus can be observed . No curative treatment is available.…”

Familial infantile bilateral striatal necrosis

“…Acquired horizontal pendular nystagmus, commonly occurring in congenital and acquired disorders of myelin, 16 appeared in Patient VI-14 at age 3.5 years and Patient VI-9 at age 5 years; optic atrophy later appeared in these two patients (age 10 and 9). The choreoathetosis progressed to hemiballismus combined with dystonic postures.…”

A 6-month trial of memantine for nystagmus and associated phenomena in oculopalatal tremor

“…VOG horizontal and vertical eye tracings from each eye were analyzed offline with custom software ( http://faculty.washington.edu/jokelly/voganalysis ) . After manual artifact rejection (i.e., blinks, drop-outs, saccades, and erroneous gaze shifts), mean amplitude, velocity, and frequency of nystagmus were computed in a stable baseline tracing ( 11 , 12 ). The mean velocity was calculated using desaccaded tracings with an array size >2,000 points.…”

Eye movement disorders and neurological symptoms in late‐onset inborn errors of metabolism

“…Ocular motor abnormalities include hypometric saccades (particularly in the horizontal plane), saccadic intrusions (square‐wave jerks), and gaze‐evoked nystagmus . Pendular nystagmus can be observed . No curative treatment is available.…”